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Thrombotic thrombocytopenic purpura

Last edited: 4/14/2026

Overview

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ dysfunction due to severe ADAMTS13 deficiency 120.

Diagnosis

  • Key Diagnostic Criteria: Microangiopathic hemolytic anemia, thrombocytopenia, neurological symptoms, and low ADAMTS13 activity (<10%) 820.
  • Recommended Tests: ADAMTS13 activity and inhibitor levels, complete blood count, peripheral blood smear, serum lactate dehydrogenase, and urinalysis 1813.
  • Differentiating TTP from HUS: Distinguish from hemolytic uremic syndrome (HUS) by ADAMTS13 activity levels and clinical presentation 119.

    • Management

  • First-Line Treatment: Therapeutic plasma exchange (TPE) is the standard initial treatment 158.
  • Adjunctive Therapies: Corticosteroids, intravenous immunoglobulin (IVIG), and rituximab for refractory cases 1712.
  • Recombinant ADAMTS13: Effective for congenital TTP and refractory cases 319.
  • Caplacizumab: Anti-von Willebrand factor (vWF) nanobody shown to increase platelet count and reduce plasma transfusion needs 9.
  • Alternative for Refractory Cases: Double-filtration plasmapheresis 18.

    • Special Populations

  • Pregnancy: Plasmapheresis can be safely performed during pregnancy 39.
  • Pediatrics: Early diagnosis and treatment are crucial; neurological outcomes can be favorable with prompt intervention 10.
  • Elderly: Management principles are similar, but comorbidities may complicate treatment 2.
  • Comorbidities: TTP can occur in association with conditions like genetic hemochromatosis and may require tailored approaches 34.

    • Key Recommendations

  • Initiate Therapeutic Plasma Exchange (TPE) promptly in suspected TTP to improve survival and outcomes (Evidence: Strong 15).
  • Include corticosteroids and IVIG in the treatment regimen for refractory cases to enhance response rates (Evidence: Moderate 712).
  • Consider recombinant ADAMTS13 for congenital TTP and refractory cases to address underlying deficiency (Evidence: Moderate 3).
  • Use caplacizumab in conjunction with TPE for rapid platelet recovery in adult patients (Evidence: Moderate 9).
  • Monitor ADAMTS13 activity and inhibitor levels regularly to guide treatment adjustments (Evidence: Expert opinion 13).

    • References

    1 Zheng XL, Al-Housni Z, Cataland SR, Coppo P, Geldziler B, Germini F et al.. 2025 focused update of the 2020 ISTH guidelines for management of thrombotic thrombocytopenic purpura. Journal of thrombosis and haemostasis : JTH 2025. link 2 Deshpande SR, Tarawneh H, Deitelzweig C, Tong J, Zhou T, Chen Y et al.. Rapid ADAMTS13 activity assays for thrombotic thrombocytopenic purpura: a systematic review and meta-analysis. Blood 2025. link 3 Scully M, Antun A, Cataland SR, Coppo P, Dossier C, Biebuyck N et al.. Recombinant ADAMTS13 in Congenital Thrombotic Thrombocytopenic Purpura. The New England journal of medicine 2024. link 4 Lee S, Jo H, Woo S, Jeong YD, Lee H, Lee K et al.. Global and regional burden of vaccine-induced thrombotic thrombocytopenia, 1969-2023: Comprehensive findings with critical analysis of the international pharmacovigilance database. European journal of haematology 2024. link 5 Wang J, Cheng F, Niu Y, Yan L, Li J, Tan B et al.. Therapeutic plasma exchange-free treatment for first-episode TTP: A systematic review. Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis 2023. link 6 Dong G, Shi FE, Yu J, Zhu J. Comparison of clinical characteristics of first-episode thrombotic thrombocytopenic purpura and TTP-like syndrome: a retrospective cohort study in a level I hematology center in China. Annals of hematology 2023. link 7 Pascual-Izquierdo C, Piñera Salmerón P, Temboury Ruiz F, Valcárcel Ferreiras D, Jiménez Hernández S, Salinas Argente R et al.. Immune thrombotic thrombocytopenic purpura: clinical suspicion and basic management in emergency departments - an expert review and consensus statement from the Spanish societies of hematology and hemotherapy (SEHH) and emergency medicine (SEMES). Emergencias : revista de la Sociedad Espanola de Medicina de Emergencias 2023. link 8 Mingot Castellano ME, Pascual Izquierdo C, González A, Viejo Llorente A, Valcarcel Ferreiras D, Sebastián E et al.. Recommendations for the diagnosis and treatment of patients with thrombotic thrombocytopenic purpura. Medicina clinica 2022. link 9 Boothby A, Mazepa M. Caplacizumab for congenital thrombotic thrombocytopenic purpura. American journal of hematology 2022. link 10 Graciaa S, Adeagbo S, Fong G, Rollins M, McElfresh P, Zerra PE et al.. Clinical features and neurological outcomes in pediatric immune-mediated thrombotic thrombocytopenic purpura: A report from a large pediatric hematology center. Pediatric blood & cancer 2022. link 11 Lane S, Shakir S. Assessing Case Fatality on Cases of Thrombosis with Concurrent Thrombocytopenia Following COVID-19 Vaccine AstraZeneca (Vaxzevria) in the United Kingdom: A Review of Spontaneously Reported Data. Drug safety 2022. link 12 Pascual-Izquierdo C, Del Rio-Garma J, de la Rubia J, Viejo A, Mingot E, Cid J et al.. Incidence, diagnosis, and outcome of immune-mediated thrombotic thrombocytopenic purpura: A nationwide survey by the Spanish registry of thrombotic thrombocytopenic purpura. Journal of clinical apheresis 2021. link 13 Mackie I, Mancini I, Muia J, Kremer Hovinga J, Nair S, Machin S et al.. International Council for Standardization in Haematology (ICSH) recommendations for laboratory measurement of ADAMTS13. International journal of laboratory hematology 2020. link 14 Simenson V, Burton J, Del Toro A, Alquist CR. Acute thrombotic thrombocytopenic purpura in Louisiana: Seasonal distribution and evaluation of an ADAMTS13 order screening protocol. Journal of clinical apheresis 2020. link 15 Borogovac A, George JN. Stroke and myocardial infarction in hereditary thrombotic thrombocytopenic purpura: similarities to sickle cell anemia. Blood advances 2019. link 16 Sadler JE. What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura. Hematology. American Society of Hematology. Education Program 2015. link 17 Lasek-Bal A, Kosarz-Lanczek K, Kazibutowska Z. Acute neurological symptoms of Moschcowitz disease - case report. Neurologia i neurochirurgia polska 2014. link 18 Karakus V, Deveci B, Kurtoğlu E. Treatment of plasmapheresis refractory thrombotic thrombocytopenic purpura with double-filtration membrane plasmapheresis. Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis 2013. link 19 George JN, Al-Nouri ZL. Diagnostic and therapeutic challenges in the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes. Hematology. American Society of Hematology. Education Program 2012. link 20 Kremer Hovinga JA, Lämmle B. Role of ADAMTS13 in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura. Hematology. American Society of Hematology. Education Program 2012. link 21 Yoo JH, Lee J, Roh KH, Kim HO, Song JW, Choi JR et al.. Rapid identification of thrombocytopenia-associated multiple organ failure using red blood cell parameters and a volume/hemoglobin concentration cytogram. Yonsei medical journal 2011. link 22 Gopal M, Cohn CD, McEntire MR, Alperin JB. Thrombotic thrombocytopenic purpura and adult onset Still's disease. The American journal of the medical sciences 2009. link 23 Zakarija A, Kwaan HC, Moake JL, Bandarenko N, Pandey DK, McKoy JM et al.. Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989-2008). Kidney international. Supplement 2009. link 24 Bennett CL, Kim B, Zakarija A, Bandarenko N, Pandey DK, Buffie CG et al.. Two mechanistic pathways for thienopyridine-associated thrombotic thrombocytopenic purpura: a report from the SERF-TTP Research Group and the RADAR Project. Journal of the American College of Cardiology 2007. link 25 Lämmle B, Kremer Hovinga JA, Alberio L. Thrombotic thrombocytopenic purpura. Journal of thrombosis and haemostasis : JTH 2005. link 26 Zakarija A, Bandarenko N, Pandey DK, Auerbach A, Raisch DW, Kim B et al.. Clopidogrel-associated TTP: an update of pharmacovigilance efforts conducted by independent researchers, pharmaceutical suppliers, and the Food and Drug Administration. Stroke 2004. link 27 Kinoshita S, Yoshioka A, Park YD, Ishizashi H, Konno M, Funato M et al.. Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura. International journal of hematology 2001. link 28 Teruya J, Styler M, Verde S, Topolsky D, Crilley P. Questionable efficacy of plasma exchange for thrombotic thrombocytopenic purpura after bone marrow transplantation. Journal of clinical apheresis 2001. link 29 Abdallah MP, Nehme ME, Azar CG. Thrombotic thrombocytopenic purpura. A case report & review of the literature. Le Journal medical libanais. The Lebanese medical journal 1999. link 30 Hori Y, Wada H, Mori Y, Shimura M, Hiyoyama K, Nakasaki T et al.. Plasma sFas and sFas ligand levels in patients with thrombotic thrombocytopenic purpura and in those with disseminated intravascular coagulation. American journal of hematology 1999. link1096-8652(199905)61:1<21::aid-ajh5>3.0.co;2-8) 31 Real E, Pastor E, Perella M, Grau E. Elective splenectomy in relapsing thrombotic thrombocytopenic purpura. Haematologica 1998. link 32 Sgarabotto D, Vianello F, Scano F, Stefani PM, Sartori R, Girolami A. Clinical and laboratoristic remission after cryosupernatant plasma exchange in thrombotic thrombocytopenic purpura. Haematologica 1998. link 33 Druschky A, Erbguth F, Strauss R, Helm G, Heckmann J, Neundörfer B. Central nervous system involvement in thrombotic thrombocytopenic purpura. European neurology 1998. link 34 Killick S, Jeffery S, Otter M, Rist C, Bevan D. Thrombotic thrombocytopenic purpura in a patient with genetic haemochromatosis, liver cirrhosis and an iron-free focus. British journal of haematology 1997. link 35 Fraser JL, Millenson M, Malynn ER, Uhl L, Kruskall MS. Possible association between the Norplant contraceptive system and thrombotic thrombocytopenic purpura. Obstetrics and gynecology 1996. link 36 Alqadah F, Zebeib MA, Awidi AS. Thrombotic thrombocytopenic purpura associated with pregnancy in two sisters. Postgraduate medical journal 1993. link 37 Ellenberger BJ, Haas L, Cundiff L. Thrombotic thrombocytopenia purpura: nursing during the acute phase. Dimensions of critical care nursing : DCCN 1993. link 38 Page Y, Tardy B, Zeni F, Comtet C, Terrana R, Bertrand JC. Thrombotic thrombocytopenic purpura related to ticlopidine. Lancet (London, England) 1991. link91383-6) 39 Watson WJ, Katz VL, Bowes WA. Plasmapheresis during pregnancy. Obstetrics and gynecology 1990. link 40 Barnert J, Behr W, Schlimok G. Thrombotic thrombocytopenic purpura without anemia. Klinische Wochenschrift 1986. link 41 Asada Y, Sumiyoshi A, Hayashi T, Suzumiya J, Kaketani K. Immunohistochemistry of vascular lesion in thrombotic thrombocytopenic purpura, with special reference to factor VIII related antigen. Thrombosis research 1985. link90180-x) 42 Garvey MB, Freedman J. Complement in thrombotic thrombocytopenic purpura. American journal of hematology 1983. link 43 Hellman RM, Jackson DV, Buss DH. Thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome in HLA-identical siblings. Annals of internal medicine 1980. link 44 Wallach HW, Oren ME, Herskowitz A. Treatment of thrombotic thrombocytopenic purpura with plasma infusions and cyclophosphamide. Southern medical journal 1979. link 45 Okuno T, Kosova L. Plasmapheresis for thrombotic thrombocytopenic purpura (TTP). Transfusion 1979. link

    Original source

    1. [1]
      2025 focused update of the 2020 ISTH guidelines for management of thrombotic thrombocytopenic purpura. — Zheng XL, Al-Housni Z, Cataland SR, Coppo P, Geldziler B, Germini F et al. Journal of thrombosis and haemostasis : JTH (2025)
    2. [2]
      Rapid ADAMTS13 activity assays for thrombotic thrombocytopenic purpura: a systematic review and meta-analysis. — Deshpande SR, Tarawneh H, Deitelzweig C, Tong J, Zhou T, Chen Y et al. Blood (2025)
    3. [3]
      Recombinant ADAMTS13 in Congenital Thrombotic Thrombocytopenic Purpura. — Scully M, Antun A, Cataland SR, Coppo P, Dossier C, Biebuyck N et al. The New England journal of medicine (2024)
    4. [4]
      Global and regional burden of vaccine-induced thrombotic thrombocytopenia, 1969-2023: Comprehensive findings with critical analysis of the international pharmacovigilance database. — Lee S, Jo H, Woo S, Jeong YD, Lee H, Lee K et al. European journal of haematology (2024)
    5. [5]
      Therapeutic plasma exchange-free treatment for first-episode TTP: A systematic review. — Wang J, Cheng F, Niu Y, Yan L, Li J, Tan B et al. Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis (2023)
    6. [6]
      Comparison of clinical characteristics of first-episode thrombotic thrombocytopenic purpura and TTP-like syndrome: a retrospective cohort study in a level I hematology center in China. — Dong G, Shi FE, Yu J, Zhu J Annals of hematology (2023)
    7. [7]
      Immune thrombotic thrombocytopenic purpura: clinical suspicion and basic management in emergency departments - an expert review and consensus statement from the Spanish societies of hematology and hemotherapy (SEHH) and emergency medicine (SEMES). — Pascual-Izquierdo C, Piñera Salmerón P, Temboury Ruiz F, Valcárcel Ferreiras D, Jiménez Hernández S, Salinas Argente R et al. Emergencias : revista de la Sociedad Espanola de Medicina de Emergencias (2023)
    8. [8]
      Recommendations for the diagnosis and treatment of patients with thrombotic thrombocytopenic purpura. — Mingot Castellano ME, Pascual Izquierdo C, González A, Viejo Llorente A, Valcarcel Ferreiras D, Sebastián E et al. Medicina clinica (2022)
    9. [9]
      Caplacizumab for congenital thrombotic thrombocytopenic purpura. — Boothby A, Mazepa M American journal of hematology (2022)
    10. [10]
      Clinical features and neurological outcomes in pediatric immune-mediated thrombotic thrombocytopenic purpura: A report from a large pediatric hematology center. — Graciaa S, Adeagbo S, Fong G, Rollins M, McElfresh P, Zerra PE et al. Pediatric blood & cancer (2022)
    11. [11]
      Assessing Case Fatality on Cases of Thrombosis with Concurrent Thrombocytopenia Following COVID-19 Vaccine AstraZeneca (Vaxzevria) in the United Kingdom: A Review of Spontaneously Reported Data. — Lane S, Shakir S Drug safety (2022)
    12. [12]
      Incidence, diagnosis, and outcome of immune-mediated thrombotic thrombocytopenic purpura: A nationwide survey by the Spanish registry of thrombotic thrombocytopenic purpura. — Pascual-Izquierdo C, Del Rio-Garma J, de la Rubia J, Viejo A, Mingot E, Cid J et al. Journal of clinical apheresis (2021)
    13. [13]
      International Council for Standardization in Haematology (ICSH) recommendations for laboratory measurement of ADAMTS13. — Mackie I, Mancini I, Muia J, Kremer Hovinga J, Nair S, Machin S et al. International journal of laboratory hematology (2020)
    14. [14]
      Acute thrombotic thrombocytopenic purpura in Louisiana: Seasonal distribution and evaluation of an ADAMTS13 order screening protocol. — Simenson V, Burton J, Del Toro A, Alquist CR Journal of clinical apheresis (2020)
    15. [15]
      Stroke and myocardial infarction in hereditary thrombotic thrombocytopenic purpura: similarities to sickle cell anemia. — Borogovac A, George JN Blood advances (2019)
    16. [16]
      What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura. — Sadler JE Hematology. American Society of Hematology. Education Program (2015)
    17. [17]
      Acute neurological symptoms of Moschcowitz disease - case report. — Lasek-Bal A, Kosarz-Lanczek K, Kazibutowska Z Neurologia i neurochirurgia polska (2014)
    18. [18]
      Treatment of plasmapheresis refractory thrombotic thrombocytopenic purpura with double-filtration membrane plasmapheresis. — Karakus V, Deveci B, Kurtoğlu E Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis (2013)
    19. [19]
      Diagnostic and therapeutic challenges in the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes. — George JN, Al-Nouri ZL Hematology. American Society of Hematology. Education Program (2012)
    20. [20]
      Role of ADAMTS13 in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura. — Kremer Hovinga JA, Lämmle B Hematology. American Society of Hematology. Education Program (2012)
    21. [21]
      Rapid identification of thrombocytopenia-associated multiple organ failure using red blood cell parameters and a volume/hemoglobin concentration cytogram. — Yoo JH, Lee J, Roh KH, Kim HO, Song JW, Choi JR et al. Yonsei medical journal (2011)
    22. [22]
      Thrombotic thrombocytopenic purpura and adult onset Still's disease. — Gopal M, Cohn CD, McEntire MR, Alperin JB The American journal of the medical sciences (2009)
    23. [23]
      Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989-2008). — Zakarija A, Kwaan HC, Moake JL, Bandarenko N, Pandey DK, McKoy JM et al. Kidney international. Supplement (2009)
    24. [24]
      Two mechanistic pathways for thienopyridine-associated thrombotic thrombocytopenic purpura: a report from the SERF-TTP Research Group and the RADAR Project. — Bennett CL, Kim B, Zakarija A, Bandarenko N, Pandey DK, Buffie CG et al. Journal of the American College of Cardiology (2007)
    25. [25]
      Thrombotic thrombocytopenic purpura. — Lämmle B, Kremer Hovinga JA, Alberio L Journal of thrombosis and haemostasis : JTH (2005)
    26. [26]
      Clopidogrel-associated TTP: an update of pharmacovigilance efforts conducted by independent researchers, pharmaceutical suppliers, and the Food and Drug Administration. — Zakarija A, Bandarenko N, Pandey DK, Auerbach A, Raisch DW, Kim B et al. Stroke (2004)
    27. [27]
      Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura. — Kinoshita S, Yoshioka A, Park YD, Ishizashi H, Konno M, Funato M et al. International journal of hematology (2001)
    28. [28]
      Questionable efficacy of plasma exchange for thrombotic thrombocytopenic purpura after bone marrow transplantation. — Teruya J, Styler M, Verde S, Topolsky D, Crilley P Journal of clinical apheresis (2001)
    29. [29]
      Thrombotic thrombocytopenic purpura. A case report & review of the literature. — Abdallah MP, Nehme ME, Azar CG Le Journal medical libanais. The Lebanese medical journal (1999)
    30. [30]
      Plasma sFas and sFas ligand levels in patients with thrombotic thrombocytopenic purpura and in those with disseminated intravascular coagulation. — Hori Y, Wada H, Mori Y, Shimura M, Hiyoyama K, Nakasaki T et al. American journal of hematology (1999)
    31. [31]
      Elective splenectomy in relapsing thrombotic thrombocytopenic purpura. — Real E, Pastor E, Perella M, Grau E Haematologica (1998)
    32. [32]
      Clinical and laboratoristic remission after cryosupernatant plasma exchange in thrombotic thrombocytopenic purpura. — Sgarabotto D, Vianello F, Scano F, Stefani PM, Sartori R, Girolami A Haematologica (1998)
    33. [33]
      Central nervous system involvement in thrombotic thrombocytopenic purpura. — Druschky A, Erbguth F, Strauss R, Helm G, Heckmann J, Neundörfer B European neurology (1998)
    34. [34]
      Thrombotic thrombocytopenic purpura in a patient with genetic haemochromatosis, liver cirrhosis and an iron-free focus. — Killick S, Jeffery S, Otter M, Rist C, Bevan D British journal of haematology (1997)
    35. [35]
      Possible association between the Norplant contraceptive system and thrombotic thrombocytopenic purpura. — Fraser JL, Millenson M, Malynn ER, Uhl L, Kruskall MS Obstetrics and gynecology (1996)
    36. [36]
      Thrombotic thrombocytopenic purpura associated with pregnancy in two sisters. — Alqadah F, Zebeib MA, Awidi AS Postgraduate medical journal (1993)
    37. [37]
      Thrombotic thrombocytopenia purpura: nursing during the acute phase. — Ellenberger BJ, Haas L, Cundiff L Dimensions of critical care nursing : DCCN (1993)
    38. [38]
      Thrombotic thrombocytopenic purpura related to ticlopidine. — Page Y, Tardy B, Zeni F, Comtet C, Terrana R, Bertrand JC Lancet (London, England) (1991)
    39. [39]
      Plasmapheresis during pregnancy. — Watson WJ, Katz VL, Bowes WA Obstetrics and gynecology (1990)
    40. [40]
      Thrombotic thrombocytopenic purpura without anemia. — Barnert J, Behr W, Schlimok G Klinische Wochenschrift (1986)
    41. [41]
      Immunohistochemistry of vascular lesion in thrombotic thrombocytopenic purpura, with special reference to factor VIII related antigen. — Asada Y, Sumiyoshi A, Hayashi T, Suzumiya J, Kaketani K Thrombosis research (1985)
    42. [42]
      Complement in thrombotic thrombocytopenic purpura. — Garvey MB, Freedman J American journal of hematology (1983)
    43. [43]
      Thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome in HLA-identical siblings. — Hellman RM, Jackson DV, Buss DH Annals of internal medicine (1980)
    44. [44]
      Treatment of thrombotic thrombocytopenic purpura with plasma infusions and cyclophosphamide. — Wallach HW, Oren ME, Herskowitz A Southern medical journal (1979)
    45. [45]
      Plasmapheresis for thrombotic thrombocytopenic purpura (TTP). — Okuno T, Kosova L Transfusion (1979)
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